Connective Tissue Disease Flashcards
SLE
Systemic lupus erythematosus
SSc
Systemic sclerosis
The range of connective tissue diseases
- Systemic lupus erythematosus (SLE)
- Antiphospholipid syndrome
- Systemic sclerosis
- Polymyositis and dermatomyositis
- Sjogren’s syndrome
- Overlap syndromes
What is lupus?
An auto-immune condition marked by inflammation, notably of the skin.
In whom does SLE typically present?
Women of child bearing age with a genetic disposition.
What is the clinical presentation of SLE?
Non-specific e.g malaise, fever, myalgia, fatigue.
Organ specific e.g lymphadenopathy, weight loss, alopecia, nail-fold infracts, non-infective endocarditis, Raynaud’s, stroke, retinal exudates, leukopenia.
Raynaud’s phenomenon
Raynaud’s affects your circulation. It causes some areas of your body, like your fingers and toes, to change colour when you’re cold or stressed.
How is SLE diagnosed?
- Anti dsDNA antibody (ANA) tests.
- Low C3 and C4 suggesting degradation of complement. - which is what causes the malfunction in the immunity pathway.
- ESR and urinalysis for protein - linking to lupus nephritis.
Note: Think of SLE when someone has a multisystem disorder and raised ESR but CRP levels normal.
If CRP is raised think of infection, serositis and arthritis.
How is SLE managed?
Topical steroids to manage flare-ups.
NSAID’s unless renal impairment.
Severe disease - immunosuppression with steroids.
What is leukopenia?
Low number of leukocytes present in the blood.
What is Libman-Sacks endocarditis (LSE)?
A form of non-bacterial endocarditis that is seen in association with systemic lupus erythematosus (SLE), antiphospholipid syndrome, and malignancies. It is one of the most common heart-related manifestations of lupus (the most common being pericarditis).
Scleroderma
Hardened skin
Systemic sclerosis
Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries.
How does systemic sclerosis present?
Scleroderma (skin fibrosis commonly of the face, hands and feet), internal organ fibrosis, microvascular abnormalities.
Hypertension - important to control.
Raynaud’s phenomenon.
Pulmonary hypertension can lead to what?
Right sided heart failure.
How is systemic sclerosis treated?
No cure.
Manage Raynaud’s with calcium channel blockers and topical GTN (Glyceryl trinitrate).
CREST syndrome
CREST syndrome is the limited cutaneous form of systemic sclerosis.
he acronym “CREST” refers to the five main features: calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.
Calcinosis
Calcinosis is the formation of calcium deposits in any soft tissue. It is a rare condition that has many different causes.
Telangiectasia
Telangiectasia is a condition in which widened venules (tiny blood vessels) cause threadlike red lines or patterns on the skin. These patterns, or telangiectases, form gradually and often in clusters. They’re sometimes known as “spider veins” because of their fine and weblike appearance.
Poly-myo-s-itis
Many muscles inflamed
Polymyositis / Dermatomyositis
Polymyositis and dermatomyositis are disorders of the body’s connective tissues, which include tendons, ligaments and the dense sheets of collagen-based tissue that cover the ends of the muscles. These diseases cause swelling weakness and tenderness in the muscles (polymyositis) and sometimes the skin (dermatomyositis).
Auto-immune.
How does dermatomyosititis present?
Myositis and skin signs:
Macular rash - Shawl sign over the back and shoulders.
Purple rash on the eyelids with oedema.
Nailfold erythema - dilated capilary loops.
General presentation of polymyositis and dermatomyosititis.
Muscular signs: Proximal muscle weakness, soreness and tenderness. Can cause dysphagia, dysphasia and respiratory weakness.
Extra-muscular signs: Fever, arthralgia, Raynaud’s, interstitial lung fibrosis, myocarditis and arrhythmias.
Note can be paraneoplastic so screen for cancers.
How is Polymyositis / Dermatomyositis diagnosed?
Muscle enzymes (ALT, AST, LDH, CK, aldolase) raised in plasma.
Muscle biopsy.
MRI shows muscle oedema in acute myositis.
EMG changes (Electromyography).
How is Polymyositis / Dermatomyositis managed?
Prednisolone and immunosupressives.
Hydroxychloroquine is used to topical skin treatment.
If you see inflamed purple eyelids think…
Gottron’s papules are a sign of what condition?
Dermatomyositis
Sjogren’s syndrome
Dry eyes and a dry mouth. The condition often accompanies other immune system disorders, such as rheumatoid arthritis and lupus.
What is the most common form of vasculitis?
Giant cell arteritis.
What is meant by limited and diffuse SSc?
This individual has skin thickening above the elbows and knees and on the chest and would be classified as having systemic sclerosis with diffuse scleroderma. An individual with skin thickening restricted to the face, forearms, hands and fingers would be said to have systemic sclerosis with limited scleroderma.
